Background:

Sjögren's syndrome (SS) has been considerably associated with anticentromere positive (ACA)-limited cutaneous subtype Systemic Sclerosis (SSc). Nonetheless, only a few studies have analysed the pattern of clinical expression and disease evolution in ACA-SSc-SS patients.

Objectives:

to compare the disease phenotype and clinical evolution of ACA-SSc-sSS patients with (a) patients with ACA-SSc and no SS (ACA-SSc) and (b) patients with primary SS (pSS), in order to verify whether the concomitant association between SS and SSc influence the patients' clinical presentation and outcome.

Results:

(a)When compared to ACA-SSc, ACA-SSc-sSS patients presented a higher frequency of arthralgias (p<0.0001), peripheral nervous involvement (p=0.006), and altered laboratory findings (p<0.0001) (i.e leukopenia, hypocomplementemia, hypergammaglobulinemia, anti-Ro/SSA, anti-La/SSB, Rheumatoid Factor), in addition to the expected increase of symptoms suggestive for glandular involvement. They also presented a lower frequency of pulmonary hypertension (p= 0.02) and sclerodactyly (p<0.0001) with 26/78 ACA-SSc-sSS patients versus 11/127 ACA-SSc patients being classified as "sine scleroderma" (p<0.0001). Noteworthy, ACA-SSc-sSS also presented a higher frequency of MALT lymphomas (p=0.0009) which occurred after a mean disease duration of 19±11 yrs, and was not correlated to skin sclerosis. (b) When compared to pSS patients, ACA-SSc-sSS patients presented a higher frequency of signs and symptoms belonging to the spectrum of SSc (p<0.0001), i.e Raynaud's phenomenon, dysphagia, teleangectasias, heart involvement and pulmonary hypertension, but also an increased prevalence of salivary glands enlargement (p<0.0001). Altered laboratory findings were also more frequent in pSS patients while no differences were observed for lymphoproliferative disorders (p=0.09)

Conclusions:

Patients with ACA-SSc-sSS presented features from both diseases: we observed that the presence of SS in ACA-SSc dramatically increased the risk of lymphoma. A careful work up is thus mandatory in the follow-up of ACA-SSc-sSS patients.